Cases reported "Eye Diseases"

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1/39. Anterior segment ischemia and sector iris atrophy: after strabismus surgery in a patient with chronic lymphocytic leukemia.

    A 69-year-old woman with chronic lymphocytic leukemia developed segmental iris atrophy and iridocyclitis after routine surgery for exotropia. Both the clinical picture and fluorescein angiogram indicated anterior segment ischemia. It is postulated that this was related to hyperviscosity of the blood caused by a high white blood cell count (114,000/cu mm). The possibility of anterior segment ischemia should be kept in mind when contemplating strabismus or retinal detachment surgery in the presence of hematologic disorders likely to increase blood viscosity. In these cases a minimal amount of surgery should be done with proper supportive therapy. strabismus surgery should be done in stages allowing for hemodynamic compensation between procedures.
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keywords = exotropia, strabismus
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2/39. The ocular manifestations of congenital infection: a study of the early effect and long-term outcome of maternally transmitted rubella and toxoplasmosis.

    PURPOSE: To study the spectrum of adverse ocular effects which result from maternally transmitted rubella and toxoplasma infection; further, to record the long-term visual and neurodevelopmental outcomes of these 2 major causes of fetal infection. STUDY DESIGN AND patients: A series of 55 patients with congenital infection have been studied prospectively on a long-term basis. The study group included a cohort of 34 cases with congenital rubella syndrome demonstrated by virus isolation, and 21 cases with a clinical diagnosis of congenital toxoplasmosis and serologic confirmation. All patients had specific disease-related ocular defects. Rubella patients were first identified during or following the last major rubella epidemic in 1963-1964, and some have been followed serially since that time. A separate study group of representative toxoplasmosis patients presented for examination and diagnosis at varying time periods between 1967 and 1991. OBSERVATIONS AND RESULTS: This study confirms that a broad spectrum of fetal injury may result from intrauterine infection and that both persistent and delayed-onset effects may continue or occur as late as 30 years after original infection. Many factors contribute to the varied outcome of prenatal infection, the 2 most important being the presence of maternal immunity during early gestation and the stage of gestation during which fetal exposure occurs in a nonimmune mother. RUBELLA: As a criteria of inclusion, all 34 rubella patients in this study exhibited one or more ocular defects at the time of birth or in the immediate neonatal period. Cataracts were present in 29 (85%) of the 34, of which 21 (63%) were bilateral. Microphthalmia, the next most frequent defect, was present in 28 (82%) of the 34 infants and was bilateral in 22 (65%). glaucoma was recorded in 11 cases (29%) and presented either as a transient occurrence with early cloudy cornea in microphthalmic eyes (4 patients), as the infantile type with progressive buphthalmos (1 patient), or as a later-onset, aphakic glaucoma many months or years following cataract aspiration in 11 eyes of 6 patients. Rubella retinopathy was present in the majority of patients, although an accurate estimate of its incidence or laterality was not possible because of the frequency of cataracts and nystagmus and the difficulty in obtaining adequate fundus examination. toxoplasmosis: Twenty-one patients with congenital toxoplasmosis have been examined and followed for varying time periods, 7 for 20 years or more. The major reason for initial examination was parental awareness of an ocular deviation. Twelve children (57%) presented between the ages of 3 months and 4 years with an initial diagnosis of strabismus, 9 of whom had minor complaints or were diagnosed as part of routine examinations. All cases in this study have had evidence of retinochoroiditis, the primary ocular pathology of congenital toxoplasmosis. Two patients had chronic and recurrent inflammation with progressive vitreal traction bands, retinal detachments, and bilateral blindness. Macular lesions were always associated with central vision loss; however, over a period of years visual acuity gradually improved in several patients. Individuals with more severe ocular involvement were also afflicted with the most extensive central nervous system deficits, which occurred following exposure during the earliest weeks of gestation. CONCLUSIONS: Although congenital infection due to rubella virus has been almost completely eradicated in the united states, the long-term survivors from the prevaccination period continue to experience major complications from their early ocular and cerebral defects. They may be afflicted by the persistence of virus in their affected organs and the development of late manifestations of their congenital infection. Congenital toxoplasmosis continues to be the source of major defects for 3,000 to 4,100 infants in the united states each year; the spectrum of defects is wide and may vary from blindness and severe mental retardation to minor retinochoroidal lesions of little consequence. Effective solutions for either the prevention or treatment of congenital toxoplasmosis have not been developed in this country but are under intensive and continuing investigation.
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ranking = 0.14500973651616
keywords = strabismus
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3/39. diplopia secondary to aniseikonia associated with macular disease.

    OBJECTIVE: To provide an explanation for diplopia and the inability to fuse in some patients with macular disease. methods: We identified 7 patients from our practices who had binocular diplopia concurrent with epiretinal membranes or vitreomacular traction. A review of the medical records of all patients was performed. In addition to complete ophthalmologic and orthoptic examinations, evaluation of aniseikonia using the Awaya New aniseikonia Tests (Handaya Co Ltd, tokyo, japan) was performed on all patients. RESULTS: All patients were referred for troublesome diplopia. Six of the patients had epiretinal membranes and 1 had vitreomacular traction. All 7 patients had aniseikonia, ranging from 5% to 18%. In 5 of the patients the image in the involved eye was larger, and in the other 2 patients it was smaller than in the fellow eye. All patients had concomitant small-angle strabismus and at least initially did not fuse when the deviation was offset with a prism. Response to optical management and retinal surgery was variable. CONCLUSIONS: aniseikonia caused by separation or compression of photoreceptors can be a contributing factor to the existence of diplopia and the inability to fuse in patients with macular disease. Concomitant small-angle strabismus and the inability to fuse with prisms may lead the clinician to the incorrect diagnosis of central disruption of fusion. Surgical intervention does not necessarily improve the aniseikonia.
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ranking = 0.29001947303233
keywords = strabismus
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4/39. Fetal alcohol syndrome.

    BACKGROUND: Fetal alcohol syndrome (FAS) describes the systemic and ocular anomalies resulting from the teratogenic effect of maternal alcohol abuse during pregnancy. It is a leading cause of preventable birth defects in the U.S. case reports: Two case reports illustrate the characteristic findings in FAS. These include growth retardation, cognitive impairment, and facial dysmorphism. Ocular signs are prevalent, including small palpebral fissure, microcornea, strabismus, myopia, astigmatism, and optic nerve hypoplasia. DISCUSSION: Fetal alcohol exposure can lead to a wide spectrum of systemic defects and vision deficits. The increasing frequency of drinking among pregnant women in recent years should call more public attention to this detrimental yet preventable syndrome. CONCLUSION: The high frequency of ocular manifestations aids in making a diagnosis of FAS, which can be challenging. Eye-care professionals can play an important role in patient management and the educational process.
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ranking = 0.14500973651616
keywords = strabismus
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5/39. Motility disturbance due to true Tenon cyst in a child with a Baerveldt glaucoma drainage implant.

    Epithelial ingrowth of the bleb cavity, a true Tenon cyst, is a rare complication of a glaucoma drainage implant. Previous cases have been associated with persistent bleb leak, and most have occurred in eyes with prior extraocular surgery. We describe a case of a true Tenon cyst causing strabismus and an elevated intraocular pressure that was successfully treated by surgical revision.
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ranking = 0.14500973651616
keywords = strabismus
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6/39. Ocular manifestations of hindbrain-related syringomyelia and outcome following craniovertebral decompression.

    PURPOSE: To analyse and evaluate associated ocular symptoms and signs in hindbrain-related syringomyelia and their response to treatment. methods: From a database of 275 patients treated in a single institution for hindbrain hernia and syringomyelia, 39 patients (14%) had ocular symptoms and signs. Only 31 patients were included in this study; the remainder were excluded due to inadequate follow-up information. All patients had confirmed evidence of hindbrain-related syringomyelia with MRI scan or CT myelogram. Treatment included craniovertebral decompression or ventriculo-peritoneal shunting. The mean follow-up was 23 months. RESULTS: In addition to the well-recognised sign of downbeat nystagmus, classically associated with foramen magnum abnormalities, a number of other ophthalmic features were identified. Symptoms included diplopia, oscillopsia, tunnel vision and difficulty in lateral gaze. Signs included nystagmus (downbeat, horizontal, rotatory, and combinations), strabismus, disc pallor, anisocoria, ptosis and field defect. patients were categorised into two groups depending on whether the ocular features were manifest at first presentation (group 1, n = 14) or developed later in the course of the disease (group 2, n = 17). The delay in diagnosis from first presentation was 5 and 6 years respectively. All patients underwent surgery. Craniovertebral decompression was performed in 13 patients in group 1 and in 15 patients in group 2. Ventriculo-peritoneal shunt was inserted in 1 patient in group 1 and in 3 patients in group 2, for the associated hydrocephalus. Following surgery, 100% of patients in group 1 and 82% of patients in group 2 had complete or partial resolution of their ocular symptoms and signs. CONCLUSIONS: The presence of unexplained ophthalmic features such as nystagmus or oscillopsia should alert one to the potential diagnosis of hindbrain-related syringomyelia. Delay in diagnosis is often associated with poorer outcome. Surgical treatment can offer excellent results for these patients.
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ranking = 0.14500973651616
keywords = strabismus
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7/39. Familial Bell's palsy in females: a phenotype with a predilection for eyelids and lacrimal gland.

    The authors report a family with familial Bell's palsy affecting seven individuals, six of whom are females. This is a distinct subtype of Bell's palsy with a predilection for juvenile females, previously reported only very rarely. In conjunction with a review of the literature, this case suggests that this phenotype carries with it a greater risk of serious complications affecting the eyelids and lacrimal gland. These carry significant functional and cosmetic implications owing to aberrant regeneration of the seventh, sixth and possibly third cranial nerves, chronicity and relapses. Clinical features include synkinesis of the eyelids with the orbicularis oris causing synkinetic ptosis, recurrent paralytic ectropion, paralysis of facial muscles of expression with dry eye, hyperlacrimation (crocodile tears), and transient strabismus. Clinically, the decision to offer surgery in place of conservative treatment should consider the natural history of chronicity and relapses often seen with this subtype of familial Bell's palsy. Botulinum toxin injections are especially versatile in managing the complications associated with this phenotype.
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ranking = 0.14500973651616
keywords = strabismus
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8/39. Refractive laser surgery in children with coexisting medical and ocular pathology.

    PURPOSE: To report the visual, refractive, and functional outcomes of photorefractive keratectomy (PRK) and of laser-assisted subepithelial keratectomy in a group of children with significant refractive error and underlying medical conditions or ocular pathology who were noncompliant with traditional management. SETTING: Nonhospital surgical facility and a hospital clinic. methods: This case series comprised 5 individual cases of anisometropic amblyopia and/or high myopia. Underlying medical and ocular conditions were as follows: upper eyelid hemangioma with oblique myopic astigmatism, Pelizaeus-Merzbacher leukodystrophy with nystagmus, klippel-trenaunay-weber syndrome with glaucoma, incontinentia pigmenti with unilateral optic nerve atrophy, and goldenhar syndrome with unilateral optic nerve hypoplasia. photorefractive keratectomy or LASEK was performed in 6 eyes of 5 patients. Age range at the time of surgery was 1.0 to 7.0 years. All procedures were performed under general anesthesia. RESULTS: Best corrected visual acuity improved by 2 lines in 2 patients and 1 line in 2 patients by 6 months after surgery. Stereopsis and/or fusional status improved in 3 patients. amblyopia treatment compliance improved in 1 patient. Alignment improved without strabismus surgery in 2 cases. A functional vision survey demonstrated a positive effect on the ability of all 5 children to function in their environment. CONCLUSION: During the period of visual cortical plasticity, refractive surgery, by eliminating the refractive component of amblyopia and by promoting fusional ability, provides considerable improvement in children, even those with underlying medical conditions associated with ocular pathology.
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ranking = 0.14500973651616
keywords = strabismus
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9/39. Hunter syndrome (MPS II-B): a report of bilateral vitreous floaters and maculopathy.

    PURPOSE: To describe bilateral vitreous opacities and maculopathy in a child with Hunter syndrome. methods: A case involving fundus examination, photography, and optical coherence tomography. medline and Embase searches found no reference to vitreous or macular abnormalities in Hunter syndrome. RESULTS: A two-year-old boy with Hunter syndrome was found to have asymmetric, bilateral, free-floating vitreous opacities that remained stable over six years. amblyopia and exotropia improved with occlusion therapy and spectacle correction. visual acuity decreased when bilateral maculopathy supervened. CONCLUSION: Screening for vitreous abnormalities and maculopathy may be important in diagnosing, treating, and explaining visual loss in Hunter syndrome.
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ranking = 0.27495131741919
keywords = exotropia
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10/39. Ocular manifestations in Fukuyama type congenital muscular dystrophy.

    We describe a child with Fukuyama type congenital muscular dystrophy (FCMD) who had several ocular manifestations since birth. These included high myopia, strabismus, nystagmus and optic atrophy of both eyes. Later he developed retinal detachment of both eyes. Our survey of 33 cases with FCMD revealed that high myopia and optic atrophy are common in FCMD. retinal detachment was reported in two cases in addition to the present one and was considered to be of developmental origin. The association of congenital muscular dystrophy with brain changes and ocular anomalies were found in FCMD, muscle, eye and brain disease (MEB) and walker-warburg syndrome (WWS). Ocular manifestations in FCMD were, in general, less severe than those in WWS or MEB. Our study suggests that FCMD, MEB and WWS are developmental abnormalities with a continual spectrum of disease severity.
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ranking = 0.14500973651616
keywords = strabismus
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